You don’t expect to break a rib while doing light housekeeping. Similarly, you wouldn’t think your worsening back pain might be caused by a type of blood cancer. However, these are among classic scenarios for patients who develop multiple myeloma, a type of blood cancer with multiple effects on your health. It disrupts your ability to fight infections, weakens once-healthy bones and potentially leads to kidney failure.
Multiple myeloma, sometimes called plasma cell myeloma or simply myeloma, is a relatively uncommon cancer, accounting for about 1% of all cancer cases.
Many patients with myeloma respond well to treatment, starting with strategically combined medications, followed by a stem cell transplant and, eventually, ongoing maintenance therapy to stave off myeloma’s return.
There is no cure for multiple myeloma. Unfortunately, relapse is the rule rather than the exception with multiple myeloma. Within a decade, the vast majority of patients will have their cancer come back in full force and again need intensive treatment. Fortunately, as therapies keep improving, remissions are getting longer.
[READ: Immunotherapy for Blood Cancer.]
What Is Multiple Myeloma?
Blood is made up of red blood cells, platelets and white blood cells. Multiple myeloma is a cancer of a specific type of white blood cells called plasma cells.
Normally, plasma cells help the body fight infections. Plasma cells are produced in the bone marrow and make antibodies, Y-shaped proteins that protect the body from disease.
With multiple myeloma, the body makes too many irregular plasma cells, overproducing an abnormal antibody called a monoclonal protein, or M protein. These proteins build up in the bone marrow, causing damage to the bones. The kidneys usually help filter these types of proteins out, but with multiple myeloma, the kidneys become overwhelmed.
The disease is called “multiple” myeloma because these cancerous plasma cells build up in the bone marrow and form multiple tumors in different bones throughout the body.
Causes and Risk Factors
For the vast majority of cases, we do not know the cause of myeloma, says Dr. Joseph Mikhael, the Scottsdale-based chief medical officer of the International Myeloma Foundation.
Here is what we do know about possible causes and risk factors for myeloma, according to Mikhael:
— Myeloma is more common in older adults, with the average diagnosis around age 69.
— Individuals of African descent are twice as likely to contract myeloma.
— Men are more likely to be diagnosed with myeloma than women
— Certain environmental exposures are connected to myeloma, such as Agent Orange, excessive radiation, smoke and possibly other chemicals.
— Myeloma does not typically run in families, but there is an increased incidence in individuals with a family member with multiple myeloma.
[READ: Worst Cancer-Causing Foods.]
Signs and Symptoms
There is no cardinal sign or symptom of myeloma, so myeloma is not usually caught early, Mikhael explains. Mikhael is also the American Society of Hematology treasurer, a professor in the Applied Cancer Research and Drug Discovery Division at the Translational Genomics Research Institute and director of Myeloma Research and Consultant Hematologist at HonorHealth Research Institute.
Patients often have general symptoms that require further evaluation, like:
— Anemia
— Bone pain (particularly in the back and ribs)
— Fractures from minimal trauma (typically in the spine)
— Bleeding or bruising easily
— Fatigue
— Weakness
— Breathing difficulties
— Nausea, loss of appetite and weight loss
— Increased thirst and frequent urination
— Frequent infections and fevers
“When myeloma is very active, it creates four common issues that we call ‘CRAB,'” says Dr. Daniel Landau, a board-certified oncologist in South Carolina and expert contributor for the Mesothelioma Center at Asbestos.com.
CRAB consists of:
— C for calcium: Myeloma causes high calcium, or hypercalcemia. Myeloma pulls calcium out of bones and into the bloodstream. Some people may have no symptoms of high calcium, while others may have loss of appetite, nausea, vomiting, weakness or brain fog.
— R for renal failure: As M proteins accumulate in the urine, it can lead to kidney damage.
— A for anemia: Anemia is a lack of healthy red blood cells. As the abnormal plasma cells grow in the bone marrow, normal red-blood-cell production is disrupted. Because red blood cells normally help supply the body with oxygen, anemia can cause weakness or shortness of breath in some people. Platelets, the blood cells responsible for clotting, can also be affected, resulting in excessive bleeding.
— B for bone: Myeloma cells secrete chemicals that can destroy bone. As calcium is depleted in the bones, they become thinner, weaker and more brittle, similar to osteoporosis. Some people experience bones that break more easily, even with minimal stress, such as picking up routine household objects. Bone pain, like unexplained and worsening back pain, can also occur.
Sometimes, a lowercase “i” is added to the CRAB acronym because infection is common with multiple myeloma. Although there are more antibiotics and vaccines to treat these infections, repeated infections are still a serious threat to those with multiple myeloma. Replacement treatment with intravenous immunoglobulin can be used for patients who have low levels of antibodies in their blood.
Diagnosis
Your doctor will need to rule out other possible conditions to provide a definitive diagnosis of multiple myeloma. You may undergo several types of testing, including the following:
— Blood and urine tests. Complete blood counts measure a patient’s level of red blood cells, white blood cells and plasma cells. Other blood tests are done to measure calcium and uric acid levels and assess kidney function. Abnormal results can then lead to more in-depth testing. Blood and urine tests called protein electrophoresis are used to measure M protein levels.
— Bone marrow biopsy. In this invasive test, a long needle is inserted into a bone, often a hip bone, to collect a bone marrow sample. You may receive a local anesthetic to numb the area before the biopsy. The sample is sent to a laboratory for analysis. Specialized lab tests can identify myeloma cells, their genetic abnormalities and how rapidly they’re dividing.
— Imaging tests. Imaging tests, such as an X-ray, MRI, CT or PET scan, are done to pinpoint the spread of myeloma and bone problems.
Diagnosing multiple myeloma includes assigning a stage number according to its extent.
Sometimes, there is a premalignant condition, before stage 1. This is called monoclonal gammopathy of undetermined significance or MGUS. Though there are no symptoms in MGUS, the M protein can be identified in the blood. MGUS screening is not done routinely for multiple myeloma surveillance because not everyone with MGUS will develop myeloma.
Slightly more advanced than MGUS is smoldering myeloma, or indolent myeloma. Someone who has progressed to smoldering myeloma has higher levels of M cells but still no symptoms. The next stage is active myeloma.
Factors that determine whether myeloma is stage 1, stage 2 or stage 3 include:
— The amount of M protein levels in the blood or urine
— Calcium levels in the blood
— The presence of anemia
— Whether bone damage exists and how advanced it is
Stages are further classified as “A” or “B” depending on the absence or presence of significant kidney damage.
Mikhael says the International Myeloma Foundation is working on earlier detection methods. “The International Myeloma Foundation participated in a large screening study in Ireland. It’s likely that we will develop a screening for myeloma like we do for breast or colon cancer.”
[SEE: Questions to Ask Your Oncologist at Your First Cancer Appointment.]
Prognosis
“It is something that is not considered to be curable, even though patients live much, much longer than they used to 10 or 15 years ago,” says Dr. Shaji Kumar, a professor and consultant in the division of hematology at Mayo Clinic in Rochester, Minnesota. “In fact, the older patients with no adverse disease characteristics may have their expected life expectancy with the current treatments.”
Kumar adds that there are roughly 36,000 new diagnoses of multiple myeloma in the United States each year, and about 12,000 people die from multiple myeloma annually in the United States.
Survival rates for multiple myeloma vary by the stage at which it’s diagnosed. Overall, the five-year survival rate is 58% according to the American Society of Clinical Oncology. For the minority of people diagnosed at an early stage, five-year survival is 79%. For people with later-stage myeloma that has spread to distant areas of the body, five-year survival is about 57%.
Not all people who have abnormal plasma cells will get myeloma. “You can detect these abnormal plasma cells and the abnormal protein they secrete, we believe, on average 20 years before myeloma comes on,” Kumar explains. “However, among the majority of people in whom you detect the abnormal protein, 80% of those people will never get myeloma. Only 20% of people will progress to the point where they actually have a cancerous condition that needs treatment.”
Treatment
Current treatment guidelines for multiple myeloma patients involve three phases.
These phases include:
— Initial (or induction) treatment
— Possible stem cell transplant
— Maintenance therapy
Each treatment phase is individualized depending on a patient’s overall health, including any organ damage or other myeloma complications and coexisting medical conditions.
Whether a patient has previously gone through myeloma treatment and relapsed, and how they responded to that treatment, is also taken into account.
Triple or quadruple-drug regimen
Some elderly or frail individuals may only receive single-drug therapy for their induction treatment. But in many cases, people receive triple or quadruple drug therapy.
The regimen includes:
— Lenalidomide. This drug enhances the immune response and inhibits the growth of myeloma cells.
— Dexamethasone. This is a steroid used to reduce inflammation and suppress the immune system to help control myeloma. It can also ease symptoms of side effects.
— Carfilzomib. This is a proteasome inhibitor that blocks protein breakdown in myeloma cells, causing them to die.
— Daratumumab. This is used in a quadruple-drug regimen, and works by finding a protein called CD38 on myeloma cells and attaching to it. This helps the body’s immune system destroy the cancer cells.
Stem cell transplant with melphalan
If your doctor determines it’s safe to have a stem cell translate, you usually have initial triple or quadruple-drug therapy followed by a stem cell transplant.
The transplant is called an autologous stem cell transplant because it uses your own stem cells — there is no outside donor. Although an autologous stem cell transplant avoids the long-term complications that can occur with a transplant using donor cells (allogenic transplant), it is still a complex procedure.
The process involves the following:
— First, stem cells are removed from your bone marrow or circulating blood and stored. “Overall, it takes about a week to collect the stem cells,” Kumar says.
— Then, you then receive high-dose chemotherapy such as melphalan to destroy the cancerous myeloma cells. During the high-dose chemotherapy, the acute phase, you need to be in the hospital or treated and monitored as an outpatient daily, Kumar says. As chemotherapy makes your blood counts drop, you may need to have transfusions. You’re also at increased risk for infection.
— Next, your stored stem cells are reinfused into your blood, to eventually mature into healthy new blood cells. It takes about two to three weeks to complete the stem cell transplant. “For most people, it’s a question of being away from home for about five to six weeks to get everything done,” Kumar says.
CAR T-cell therapy
CAR T-cell therapy involves changing the genes in certain immune cells to help them fight cancer. This therapy is used in other conditions sometimes, but in multiple myeloma, they target the BCMA protein because it is found in high amounts on the surface of myeloma cells.
The therapy is performed in three phases:
— Collecting T-cells: T-cells are taken from the blood using a leukapheresis machine. The blood goes through the machine to remove the T-cells, and then the rest of the blood goes back into the body. This usually takes a few hours.
— Genetic alteration and multiplication: The T-cells are sent to a lab, where they are changed to have receptors on their surface that help them find and attach to cancer cells. The lab then makes more of these altered T-cells over several weeks.
— Infusing CAR T-cells: The individual receives chemotherapy to prepare their immune system for the CAR T-cells. Then, the new CAR T cells are put back into the blood, where they find and attack the cancer cells.
Side effects
As with transplant and high-dose chemotherapy, the initial triple or quadruple-drug therapy may cause a variety of side effects, depending on the specific medications. Side effects often include:
— Nausea, vomiting and diarrhea
— Weight gain, increased blood sugar and changes in mood or sleep due to the steroids, like dexamethasone.
— Fatigue, reduced blood count, increased risk of blood clots or nerve damage from immune-modifying drugs or proteasome inhibitors, like carfilzomib.
Managing side effects like these to help you better tolerate medications and transplant is an important part of your myeloma treatment. Once initial therapy is completed, response rates are encouraging. “Between the treatments that we have — transplant and combination therapy — between 95% and 97% of patients will respond,” Kumar says.
Maintenance therapy
Typically, patients stay on a lower dose of a drug once they’re in remission. Other drugs or combinations may be used.
For maintenance thearpy, the following medications are often used:
— Lenalidomide
— Bortezomib
— Ixazomib
Remission
Remission is the goal for multiple myeloma treatment. Remission means you no longer have myeloma symptoms or detectable signs of disease in your blood, urine or bone marrow. Treatment response may be complete or partial and remission can be permanent or temporary.
Some patients will experience shorter-than-average remissions, while others may stay in remission up to 10 years or even longer.
When in remission, it’s important to keep up with some lifestyle changes:
— Due to the strain on the kidneys caused by multiple myeloma, your doctor may recommend a kidney diet.
— Due to the bone breakdown from multiple myeloma, you’ll need to be careful to avoid lifting heavy objects.
— You may need to take supplements, Kumar says. “It’s recommended that patients take calcium and vitamin D supplements to help with bone disease.”
“Most of the patients we see are able to do the majority of the things that they are accustomed to with little limitations, especially once the disease is in remission,” Mikhael adds.
Complications
Myeloma complications can be formidable. In addition to managing cancer, many patients contend with associated bone and kidney conditions.
Common complications include:
— Compression fractures due to weakened and brittle bones
— Kidney failure, which may require dialysis several times per week. Dialysis helps to filter the blood, removing waste, salt and excess fluid from the body and controlling blood pressure, which are essential kidney functions.
— Anemia, which may require occasional blood transfusion to restore red blood cells to the proper levels, or your doctor may recommend a medication called as erythropoietin to stimulate the bone marrow to produce more red blood cells.
Health Care Providers
As you undergo diagnosis and treatment for multiple myeloma, members of your health care team may include several of these specialists:
— Oncology nurse
— Radiologist
— Orthopedic surgeon
— Neurologist.
— Nephrologist (kidney specialist)
— Pain management specialist
— Oncology pharmacist
Dedicated myeloma centers are also useful to offer staff-wide expertise in managing myeloma and access to the latest treatments, along with specialists who can be immediately consulted when people have myeloma-related complications like spinal fractures. Research is a priority.
In some cases, participating in a clinical trial can offer you access to state-of-the art treatments awaiting approval by the FDA.
The Multiple Myeloma Research Foundation has an online search tool to locate U.S. treatment centers with a focus on multiple myeloma.
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A Guide to Multiple Myeloma: Symptoms, Treatment and Prognosis originally appeared on usnews.com
Update 09/11/24: This story was previously published at an earlier date and has been updated with new information.
